Overview
Definition:
Eosinophilic Esophagitis (EoE) is a chronic, immune-mediated esophageal disease characterized by symptoms of esophageal dysfunction and eosinophil-predominant inflammation in the esophagus, diagnosed by endoscopic biopsy showing ≥15 eosinophils per high-power field (eos/hpf).
Epidemiology:
EoE is a leading cause of food-responsive esophageal inflammation and dysphagia in children and adults
Prevalence is estimated to be 1 in 1000 to 1 in 5000 children, with increasing incidence reported globally
It is more common in males and individuals with a history of allergic comorbidities such as asthma, eczema, and allergic rhinitis.
Clinical Significance:
EoE significantly impacts quality of life due to symptoms like dysphagia, vomiting, abdominal pain, and feeding difficulties in children
Untreated or poorly managed EoE can lead to esophageal strictures, food impaction, and malnutrition, making accurate diagnosis and effective management crucial for pediatric residents preparing for DNB and NEET SS examinations.
Clinical Presentation
Symptoms:
In infants and young children: feeding intolerance, vomiting, failure to thrive, abdominal pain
In older children and adolescents: dysphagia (most common), food impaction, chest pain, vomiting, abdominal pain, regurgitation.
Signs:
Physical examination is often unremarkable
However, signs of atopy (eczema, allergic rhinitis) may be present
In severe, long-standing cases, esophageal strictures may lead to palpable masses on abdominal examination
Failure to thrive can be seen in infants with significant feeding difficulties.
Diagnostic Criteria:
The 2017 AASLD (American Academy of Allergy, Asthma & Immunology) guidelines define EoE by the presence of: 1
Symptoms of esophageal dysfunction
2
≥15 eos/hpf in esophageal biopsies taken from at least two different levels of the esophagus, in the absence of other causes of esophageal eosinophilia (e.g., proton pump inhibitor response, parasitic infection, medication-induced esophagitis)
3
No significant response to PPI therapy (typically 8 weeks of PPIs is attempted to rule out eosinophilic esophagitis with PPI-responsiveness).
Diagnostic Approach
History Taking:
Detailed history of feeding difficulties, vomiting, abdominal pain, chest pain, dysphagia, food impaction
Inquire about allergic comorbidities (asthma, eczema, allergic rhinitis, food allergies), family history of atopy or EoE, and response to previous dietary eliminations or medications.
Physical Examination:
Focus on general growth parameters (height, weight, BMI)
Assess for signs of atopy (skin lesions, nasal polyps, conjunctivitis)
Palpate abdomen for tenderness or masses
Perform respiratory examination to assess for wheezing suggestive of asthma.
Investigations:
Upper endoscopy with esophageal biopsies is the gold standard
Biopsies should be taken from both the proximal and distal esophagus, ideally from multiple sites (at least 6 biopsies)
Histopathological examination looking for ≥15 eos/hpf
Allergy testing (skin prick tests or serum IgE) can identify specific triggers
Esophageal manometry may reveal dysmotility but is not diagnostic for EoE
Barium swallow can show strictures or dysmotility but is less sensitive than endoscopy.
Differential Diagnosis:
Gastroesophageal reflux disease (GERD) - PPI trial helps differentiate
Eosinophilic gastroenteritis
Eosinophilic proctitis
Celiac disease
Infections (e.g., Candida esophagitis)
Achalasia
Esophageal strictures due to other causes
Functional dysphagia.
Management
Initial Management:
Identification and removal of trigger foods
Dietary elimination is a cornerstone of therapy
Empiric trial of proton pump inhibitors (PPIs) is often initiated first to rule out PPI-responsive esophageal eosinophilia (PPI-REE).
Medical Management:
Topical Steroids: Swallowed fluticasone propionate (220 mcg, 2 puffs, twice daily, swallowed without water) or budesonide (dissolved in a viscous vehicle, 1 mg, twice daily, swallowed)
Aim is to reduce esophageal eosinophil counts and improve symptoms
Doses can be adjusted based on response
Duration of therapy is typically long-term.
Dietary Management:
Elemental Diet: Exclusive enteral nutrition (EEN) using a defined formula (e.g., amino acid-based formula) for 2-6 weeks has a high remission rate (80-90%)
This is often considered the most effective treatment, especially for severe or refractory cases
Following remission, reintroduction of foods one by one can help identify specific triggers for step-up diets (e.g., 4-food elimination diet, 6-food elimination diet).
Supportive Care:
Nutritional support is vital, especially in infants and children with failure to thrive or feeding difficulties
Education for patients and families regarding the chronic nature of EoE, trigger avoidance, and medication adherence is crucial
Long-term follow-up with a multidisciplinary team (pediatric gastroenterologist, allergist, dietitian) is recommended.
Complications
Early Complications:
Food impaction is a common and potentially emergent complication requiring endoscopic removal
Esophageal strictures can develop over time due to chronic inflammation.
Late Complications:
Fibrosis and stricture formation can lead to significant dysphagia and an increased risk of esophageal perforation during dilation procedures
Malnutrition and failure to thrive in pediatric patients.
Prevention Strategies:
Adherence to prescribed dietary modifications or topical steroid therapy
Regular endoscopic surveillance in patients with severe disease or a history of strictures
Prompt management of food impactions.
Prognosis
Factors Affecting Prognosis:
Early diagnosis and effective management, patient adherence to treatment, presence of comorbidities like asthma, and severity of esophageal fibrosis influence prognosis.
Outcomes:
With appropriate management, most children with EoE can achieve symptom remission and reduced esophageal eosinophilia, leading to improved quality of life
However, EoE is often a lifelong condition requiring ongoing management.
Follow Up:
Regular follow-up with a pediatric gastroenterologist and allergist is essential
This typically includes periodic symptom assessment and may involve repeat endoscopies with biopsies to monitor treatment efficacy and disease activity, particularly during dietary interventions or after changes in therapy
Monitoring for potential long-term complications like strictures is crucial.
Key Points
Exam Focus:
Distinguish EoE from GERD based on PPI trial and biopsy findings (≥15 eos/hpf)
Understand the mechanisms and efficacy of elemental diet (most effective) vs
topical steroids
Recognize symptoms of food impaction and management strategies.
Clinical Pearls:
Always biopsy the esophagus during endoscopy for suspected EoE, even if visually normal
Topical steroids should be swallowed and not inhaled
Elemental diet requires significant family commitment and dietitian support
Consider food allergy testing to guide dietary elimination.
Common Mistakes:
Misdiagnosing EoE as GERD and relying solely on PPIs without biopsy
Insufficient biopsy sampling (less than 6 biopsies from proximal and distal esophagus)
Not considering EoE in children with unexplained vomiting or feeding difficulties
Inadequate follow-up and monitoring of treatment response.